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Table of Contents
CASE REPORT
Year : 2017  |  Volume : 14  |  Issue : 3  |  Page : 188-189

Postpartum hypernatremic encephalopathy with “Wine Glass Sign” on magnetic resonance imaging


1 Department of Neurology, Indraprastha Apollo Hospital, New Delhi, India
2 Department of Nephrology, Indraprastha Apollo Hospital, New Delhi, India
3 Department of Obstetrics and Gynaecology, Indraprastha Apollo Hospital, New Delhi, India
4 Internal Medicine, Ram Manohar Lohia Hospital, New Delhi, India

Date of Web Publication27-Oct-2017

Correspondence Address:
Vinit Suri
Department of Neurology, Indraprastha Apollo Hospital, Sarita Vihar, Mathura Road, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/am.am_3_17

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  Abstract 


We report a 28-year-old postpartum female with acute delirium, agitation, confusion, visual hallucinations, and limb weakness. Investigations revealed severe hypernatremia, hyperuricemia, rhabdomyolysis, acute kidney injury, normal serum and urine osmolarity, normal urine spot sodium level, and normal serum antidiuretic hormone level. Magnetic resonance imaging of brain revealed hyperintensities in bilateral corticospinal tracts showing the classical “Wine Glass sign” suggesting hypernatremia-related osmotic demyelination. Patient improved with gradual correction of hypernatremia. We would like to highlight the importance of timely recognition and appropriate treatment of this rare condition which is potentially fatal and may also lead to severe permanent neurological deficits.

Keywords: Hypernatremia, osmotic demyelination syndrome, postpartum, Wine Glass sign


How to cite this article:
Suri V, Jasuja S, Suri N, Saini R, Naga MK, Suri K. Postpartum hypernatremic encephalopathy with “Wine Glass Sign” on magnetic resonance imaging. Apollo Med 2017;14:188-9

How to cite this URL:
Suri V, Jasuja S, Suri N, Saini R, Naga MK, Suri K. Postpartum hypernatremic encephalopathy with “Wine Glass Sign” on magnetic resonance imaging. Apollo Med [serial online] 2017 [cited 2019 Jun 27];14:188-9. Available from: http://www.apollomedicine.org/text.asp?2017/14/3/188/217366




  Introduction Top


Hypernatremia is diagnosed when sodium levels in serum exceed 145 mEq/L and can result in neurological manifestations including seizures, encephalopathy, rhabdomyolysis as well as serious autonomic and brain stem dysfunction. Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are frequently associated with hyponatremia or its rapid correction. Hypernatremia has also been associated with osmotic demyelination with distinctly different imaging characteristics on magnetic resonance imaging (MRI). Postpartum hypernatremia is a recently described entity where severe hypernatremia occurs in the postpartum period and presents as an encephalopathy with rhabdomyolysis with diffuse white matter hyperintensities suggestive of osmotic demyelination. We present a young female with postpartum hypernatremia with typical clinical and radiological features.


  Case Report Top


A 28-year-old primigravida delivered a female infant through a full-term normal vaginal delivery conducted at a tertiary care hospital. She developed hypothyroidism in the first trimester and pregnancy-induced-hypertension requiring antihypertensive medication for 2 months in the second trimester. Patient was discharged on 3rd postpartum day. Neither ritualistic feeding nor any ritualistic dehydration was conducted at home. Patient developed 2–3 small volume loose motions unaccompanied by any vomiting or fever on 5th postpartum day followed next day by an agitated and confused state with visual hallucinations. Investigations revealed severe hypernatremia (187 mEq/L), elevated creatine phosphokinase (CPK) (2213 IU/L), elevated creatinine (2.4 mg/dl), and leukocytosis (11,190/cumm). Patient was transferred to our institute where examination revealed an agitated confused patient with subtle weakness of all four limbs with bipyramidal signs. MRI brain revealed bilateral symmetrical T2-weighted (T2W)/fluid attenuation inversion recovery (FLAIR) hyperintensities with restricted diffusion involving the lateral thalamus, pons, mid brain, splenium of corpus callosum, and centrum semiovale in a “Wine Glass” pattern [Figure 1] consistent with osmotic demyelinating process. Repeat investigations revealed serum sodium 178 mEq/L, urine spot sodium 47 mmol/L (normal >20 mmol/L), urine spot osmolarity 0.751 (normal 0.5–1.4), and serum osmolarity 0.411 osmol/kg (normal 0.28–0.301). Serum antidiuretic hormone (ADH) level was 4 (normal 1–5pcg/mL). Antinuclear antibody was negative, and serum C3 and C4 levels were normal. Routine hematological and biochemical investigation were normal except creatinine (2.4 mg/dl), urea (134 mg/dl), lactate dehydrogenase (918 IU/L), and CPK (2213 IU/L). Cerebrospinal fluid analysis was normal. Patient was managed with gradual correction of hypernatremia by <10 mEq/L/day with oral and intravenous fluids. Encephalopathy and quadriparesis worsened over 4–5 days followed by plateauing for 4–5 days followed by resolution of encephalopathy and improved motor power to 5/5 over 2 weeks. Repeat MRI brain performed 2 weeks later revealed significant resolution of the bilateral hyperintensities [Figure 2]. Serological parameters improved gradually and by 10 days creatinine improved to 0.6 mg/dl, urea to 31 mg/dl, and CPK to 313 IU/L.
Figure 1: Magnetic resonance imaging brain images (a) depicting classic “Wine Glass sign” on coronal T2-weighted images. (b) Axial fluid-attenuated inversion recovery images revealing hyperintense signals in internal capsule and corpus callosum. (c) Axial fluid-attenuated inversion recovery images revealing hyperintense signals in centrum semiovale and subcortical white matter

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Figure 2: Magnetic resonance imaging brain images showing resolution of previous findings 2 weeks after initial imaging (a) coronal T2-weighted images. (b and c) Axial fluid-attenuated inversion recovery images

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  Discussion Top


Common causes of postpartum neurological symptoms are eclampsia, cerebral venous sinus thrombosis, and posterior reversible leukoencephalopathy syndrome. In addition, multiple dyselectrolytemias lead to neurological symptoms. Hyponatremia causes mental confusion, seizures, drowsiness, obtundation, and if severe can lead to a metabolic coma. Rapid correction of chronic hyponatremia can lead to osmotic demyelination syndrome with lesions in the central pons, called CPM or in extrapontine tissues, called EPM. Hypernatremia causes a similar clinical although a different MRI picture.

The causes of hypernatremia include diarrhea, diuresis, decreased water intake, excessive sweating, exogenous salt intake, ADH deficiency, vomiting, and various metabolic disorders. Postpartum hypernatremia is a rare disorder which has been reported in a few case reports and one case series.[1],[2],[3],[4] Postpartum hypernatremia may occur due to ritualistic feeding and dehydration practiced in some communities in India. In addition, postpartum hypothalamic dysfunction leading to lack of thirst sensation despite the presence of dehydration and hypernatremia can lead to a central adipsic hypernatremia. A partial ADH secretion defect may also be unmasked during pregnancy due to the enhanced peripheral breakdown by vasopressinase. Clinical features of postpartum hypernatremia include quadriparesis, delirium, disorientation, irrelevant speech, dysarthria, ataxia, seizures, rhabdomyolysis, hyperuricemia, acute kidney injury, encephalopathy, and coma. The diagnosis of postpartum hypernatremia is based on the presence of elevated serum sodium level, and the presence of resultant osmotic cerebral demyelination requires evaluation with a MRI. MRI abnormalities include bilateral T2 Weighted/FLAIR hyperintensities involving corpus callosum, especially splenium, with the involvement of internal capsule, cerebellar peduncle, pons, and hippocampus, and this picture of bilateral symmetrical hyperintensities in corticospinal tracts leads to a picture of “Wine Glass” pattern of hyperintensities which has previously been described in amyotrophic lateral sclerosis and certain leukodystrophies.[3] Management includes early recognition and gradual correction of hypernatremia by oral and intravenous free water gradually by <10 mEq/L/day. Overall prognosis for neurological recovery is difficult to quantify due to the dearth of literature. In one case series, mortality was 36.36%,[1] while many of the remaining patients had residual neurological deficits.

Timely diagnosis of the same and adequate correction will lead to improvement and avoid lasting complications and neurodeficits. Obstetricians need to be aware of this pregnancy and postpartum-induced treatable disorder since they would usually be the first contact in these pregnant and postpartum patients and need to act urgently to prevent morbidity and mortality.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Naik KR, Saroja AO. Seasonal postpartum hypernatremic encephalopathy with osmotic extrapontine myelinolysis and rhabdomyolysis. J Neurol Sci 2010;291:5-11.  Back to cited text no. 1
[PUBMED]    
2.
Schrier RW. Systemic arterial vasodilation, vasopressin, and vasopressinase in pregnancy. J Am Soc Nephrol 2010;21:570-2.  Back to cited text no. 2
[PUBMED]    
3.
Saroja AO, Naik KR, Mali RV, Kunam SR. 'Wine glass' sign in recurrent postpartum hypernatremic osmotic cerebral demyelination. Ann Indian Acad Neurol 2013;16:106-10.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Bhatia S, Kapoor AK, Sharma A, Gupta R, Kataria S. Cerebral encephalopathy with extrapontine myelinolysis in a case of postpartum hypernatremia. Indian J Radiol Imaging 2014;24:57-60.  Back to cited text no. 4
[PUBMED]  [Full text]  


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